No abstract available.
Child* ; Humans

No abstract available.
Carcinoma, Squamous Cell* ; Lung Neoplasms* ; Lung* ; Small Cell Lung Carcinoma*

No abstract available.
Hepatitis, Chronic* ; Interferon-alpha* ; Killer Cells, Natural* ; Prednisolone*

Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

BACKGROUNDS: Obstructive sleep apnea syndrome(OSA) can divided into two groups, positional (PP) and non-positional(NPP) obstructive sleep apnea syndrome, according to the body position while sleeping. In this study, we evaluated the differences of anthropometirc data and polysomnographic recordings between the two types of sleep apnea syndrome. MATERIALS: Fifty patients with OSA were divided two groups by Cartwright's criteria. The supine respiratory disturbance index (RDI) was at least two times higher than the lateral RDI in the PP group, and the supine RDI was less than twice the lateral RDI in the NPP group. This patients underwent standardized polysomnographic recordings. The anthropometirc data and polysomnographic data were analyzed, statistically. RESULTS: Of all 50 patients, 30% were found to be positional OSA. BMI was significantly higher in the PP group(p<0.05). Total sleep time was significantly longer in the PP group (350.6±46.0min, p<0.05). Sleep efficiency was high in the PP group(89.6± 6.4%, 85.6±9.9%, p<0.05). Deep sleep was significantly higher and light sleep was lower in the PP group than in the NPP group but no difference was observed in REM sleep between the two groups. Apnea index(AI) and RDI were significantly lower(17.0±10.6, 28.5±13.3, p<0.05) and mean arterial oxygen saturation was higher in the PP group(92.7 ±1.8%, p<0.05) than in the NPP group. CONCLUSION: Body position during sleep has a profound effect on the frequency and severity of breathing abnormalities in OSA patients. A polysomnographic evaluation for suspected OSA patients must include monitoring of the body position. Breathing function in OSA patients can be improved by controlling their obesity and through postural therapy.
Apnea ; Humans ; Obesity ; Oxygen ; Polysomnography ; Respiration ; Sleep Apnea Syndromes ; Sleep Apnea, Obstructive* ; Sleep, REM

The clinical triad of relapsing iritis, ulcers of the mouth and genitalia was first described in 1939 by Hulusi Behcet. This entity, originally confined to the above triad of symptoms appears to be systemic disease manifested by skin lesion, thrombophlebitis, neurologic, cardiovascular or visceral symptoms. The vascular involvement in Behcet's syndrome has been reported since Mischima first described a case in 1961, four types of vascular lesion are freuqnetly observed most commonly on the inferior or superior vena cava. Treatments consist of anticoagulation and administering oral steroids. We report a case of SVC obstruction in 36 years old female patient with Behcet's syndrome.
Adult ; Behcet Syndrome* ; Female ; Genitalia ; Humans ; Iritis ; Mouth ; Skin ; Steroids ; Thrombophlebitis ; Ulcer ; Vena Cava, Superior

Mechanical obstruction of the common hepatic duct includes the following causes; choledocholithiasis, sclerosis, cholangitis, pancreatic carcinoma, cholangiocarcinoma, postoperative stricture, primary hepatic duct carcinoma, enlarged cystic duct lymph nodes, and metastatic nodal involvement of the porta hepatis. Partial mechanical obstruction of the common hepatic duct caused by impaction of stones and inflammation surrounding the vicinity of the neck of the gallbladder had been reported on the “syndrome del conducto hepatico” in 1948 by Mirizzi. Nowadays, this disease was named by Mirizzi syndrome. Mrizzi syndrome is a rare entity of common hepatic duct obstruction that results from an inflammatory response secondary to a gallstone impacted in the cystic duct or neck of the gallbladder. It results from an almost parallel course and low insertion of the cystic duct into the common hepatic duct. In a variant of Mirizzi's syndrome, the cause of the common hepatic duct obstruction was a primary cystic duct carcinoma rather than gallstone disease. A 71-year-old man was admitted with a four-day history of right upper quadrant abdominal pain. Past medical history was unremarkable. On physical examination, the patient had a temperature of 38℃, icteric sclera and right upper quadrant tenderness. Pertinent laboratory findings included WBC 18,000/cm3; albumin 2.6 g/dl (normal 0-1) with the direct bilirubin, 4.4 mg/dl (normal 0-0.4). Ultrasonography revealed a dilated extrahepatic biliary tree. ERCP showed that the superior margin was angular and more consistent with a calculus causing partial CHD obstruction (Mirizzi syndrome). At surgery a diseased gallbladder containing calculi was found. In addition, there was two calculi partially eroding through the proximal portion of the cystic duct and compressing the common hepatic duct. A cholecystectomy and excision of common bile duct was performed, with Roux-en-Y hepaticojejunostomy. The postoperative course was uneventful.
Abdominal Pain ; Aged ; Biliary Tract ; Bilirubin ; Calculi ; Cholangiocarcinoma ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis ; Cholecystectomy ; Choledocholithiasis ; Common Bile Duct ; Constriction, Pathologic ; Cystic Duct ; Cytochrome P-450 CYP1A1 ; Gallbladder ; Gallstones ; Hepatic Duct, Common ; Humans ; Inflammation ; Jaundice, Obstructive ; Lymph Nodes ; Mirizzi Syndrome* ; Neck ; Physical Examination ; Sclera ; Sclerosis ; Ultrasonography

BACKGROUND: The effects of chemotherapy on pulmonary function are mainly a reduced diffusion capacity and a restrictive ventilatory impairment. Exercise can expose cardiovascular and pulmonary abnormalities not evident at rest. Exercise related cardiopulmonary function is important in patients with malignant disease as a determinant of quality of life. We performed this study to evaluate the changes of body composition and cardiopulmonary exercise performance of patients with locally advanced, non-small cell, lung cancer (NSCLC) before and after chemotherapy. METHODS: We evaluated resting pulmonary function, body composition, physiologic performance status, and cardiopulmonary exercise function in 11 patients with locally advanced NSCLC, at diagnosis and prior to the fourth cycle of chemotherapy. RESULTS: After chemotherapy, 4 patients (36.4%) showed partial response and 7 (63.4%) had stable disease. After chemotherapy, diffusion capacity of the lung for carbon monoxide was reduced (89.7+/-34.1%, vs. 71.9+/-20.5%) but not significantly. There were no significant changes in body composition or the state of physiologic performance after chemotherapy. There was a significant impairment of cardiopulmonary exercise tolerance in patients with NSCLC, evidenced by a reduction of maximal oxygen uptake (VO2max, ml/kg/min, 17.9+/-2.6 : 12.6+/-6.1, <0.05) and O2 pulse (O2 pulse, ml/beat, 7.0+/-1.7, 5.2+/-2.1, <0.05). CONCLUSION: Systemic chemotherapy resulted in a loss of cardiopulmonary exercise function in patients with locally advanced NSCLC within the short-term period, but not a physiologic change of body composition within the same period.
Body Composition ; Carbon Monoxide ; Carcinoma, Non-Small-Cell Lung* ; Diagnosis ; Diffusion ; Drug Therapy* ; Exercise Test* ; Exercise Tolerance ; Humans ; Lung ; Lung Neoplasms ; Oxygen ; Quality of Life

BACKGROUND AND OBJECTIVES: Cardiac troponin T (cTnT) has been used as a very sensitive marker of cardiac injury caused by ischaemia, myocarditis, and cardiomyopathy. After cardiac injury, the fetal cTnT isoform expression in the heart and serum cTnT increases. To investigate the increased levels of serum cTnT, and the expression of fetal cTnT isoform in the heart, that can predict myocardial injury, we measured serum cTnT levels and the fetal cTnT isoform expression at various time points during the early phase of myocardial toxicity induced by adriamycin (ADR) in rat. MATERIALS AND METHODS: Male Sprague-Dawley rats were injected, intraperitoneally, with ADR (5 mg/kg) twice a week for 2 weeks. Control rats were injected with saline. Serum cTnT levels were measured by ELISA. The ratio of fetal/adult (F/A) cTnT isoform expression (%) was semi-quantified by RT-PCR using total RNA from frozen hearts. RESULTS: Serum cTnT levels did not increase by 1 week after ADR injection, but increased significantly after 2 weeks. The ratio of F/A cTnT in the heart significantly increased from day 1, peaked at 1 week and persisted until the end of 2 week. CONCLUSION: The expression of the fetal cTnT isoform occurred from 1 day after ADR injection when the serum cTnT levels were still normal. Although the serum cTnT level is a very sensitive, and an early marker, of cardiac damages, the fetal cTnT isoform expression in the endomyocardial biopsy specimen may be a more sensitive and an earlier marker in the ADR-induced myocardial damage.
Animals ; Biopsy ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Doxorubicin ; Enzyme-Linked Immunosorbent Assay ; Heart ; Humans ; Male ; Myocarditis ; Rats* ; Rats, Sprague-Dawley ; RNA ; Troponin T* ; Troponin*

No abstract available.
Alveolitis, Extrinsic Allergic* ; Hypersensitivity*